SOD1 / Superoxide Dismutase抗体

SOD1 / Superoxide Dismutase抗体产品信息免疫原 :  Recombinant Human SOD1 / Superoxide Dismutase protein (Catalog#11727-H07E)
 
Reagents :  FITC-conjugated mouse monoclonal antibody 
Clone ID : 11
抗体宿主 : Mouse IgG2b
Concentration : 5 μl/Test, 0.1 mg/ml
缓冲液 : Aqueous solution containing 0.5% BSA and 0.09% sodium azide 
制备方法 :  This antibody was produced from a hybridoma resulting from the fusion of a mouse myeloma with B cells obtained from a mouse immunized with purified, recombinant Human SOD1 / Superoxide Dismutase (rh SOD1 / Superoxide Dismutase; Catalog#11727-H07E; NP_000445.1; Ala2-Gln154) and conjugated with FITC under optimum conditions, the unreacted FITC was removed.
SOD1 / Superoxide Dismutase抗体 Background
Superoxide dismutase [Cu-Zn], also known as superoxide dismutase 1 and SOD1, is an enzyme that in humans is a cytoplasm protein which belongs to theCu-Zn superoxide dismutase family. SOD1 binds copper and zinc ions and is one of three isozymes responsible for destroying free superoxide radicals in the body. SOD1 is a soluble cytoplasmic and mitochondrial intermembrane space protein, acting as a homodimer to convert naturally occurring, but harmful, superoxide radicals to molecular oxygen and hydrogen peroxide. SOD1 has a tendency to form fibrillar aggregates in the absence of the intramolecular disulfide bond or of bound zinc ions. These aggregates may have cytotoxic effects. Zinc binding promotes dimerization and stabilizes the native form. Defects in SOD1 are the cause of amyotrophic lateral sclerosis type 1 (ALS1) which is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Death usually occurs within 2 to 5 years. The disease is inherited in 5-10% of cases leading to familial forms.
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